Regarding German haemophiliacs, we analyzed the clinical data of 40 severe affected cases, obtained from ESCHQoL program. As the ESCHQoL program includes also haemophiliacs from other European countries including old patients up to 88 years, we have collected only those German severe affected cases of haemophilia who match the Yemeni haemophiliacs regarding their ages. The age of collected patients varied from 3 years to 29 years with a mean of 12.7 years. Haemophilia A and Hemophilia B cases were distributed with a proportion of 80% to 20% of total cases, respectively.
The general characteristics of patients are given in Table 22. A detailed table is given in the appendix.
Table 22: General characteristics of German haemophiliacs
Total number of patients 40
Severe cases 40
Mild and moderate cases 0
Age
Min-Maximal 3years - 29 years
Mean 12.7 years Median 10 years Type of Haemophilia A 32 cases B 8 cases 6.6.2.2 Clinical manifestations
Age at first bleeding varied from 0 to 39 months, with an average of 10 months. The first bleeding site was mainly a joint bleed. The number of bleedings in the last 6 months varied from 0 to 12 with an average of 2.5 bleeds. The most affected joint was knee followed by ankle, as shown in Figure 13.
Figure 12: Frequency of joint bleeding in German patients
Regarding other clinical manifestations rather than joint bleeding, only one patient has had an intracranial bleeding (ICB) during the last 6 months. The clinical manifestations among German haemophiliacs during last 6 months are shown in Figure 14.
Figure 13: Clinical manifestations among German haemophiliacs
6.6.2.3 Concomitant diseases and complications
Concerning chronic diseases, one case was affected by hepatitis C virus (HCV), 2 cases with HIV, one case with joint atrophy and one case was complicated with anaemia. 37,5 7,5 5 2,5 0 10 20 30 40 50 60 70 80 90 100 Joint bleeding GIT bleeding Hematuria ICB C a se s i n % Clinical manifestation
Figure 14: Complicated German cases
Five cases developed inhibitors during therapy with factor concentrates. They represented 12.5% of total cases.
6.6.2.4 Treatment modality
All cases were on prophylactic treatment, most of them with secondary prophylaxis, and almost all of them, except one case, receive home treatment.
Figure 15: Type of prophylaxis provided to German haemophiliacs
During the last 6 months period, factor concentrates were given to all of 40 patients. In total, an amount of 4,009,770 IU of factor concentrates were consumed during the last 6 months with a mean of 100,244 IU per patient. The majority of cases, as shown in Figure 17, were treated by recombinant factor concentrates. No case was treated with FFP, Cryoprecipitate or DDAVP.
2 1 1 0 1 2 3 HIV HCV Anemia N o of ca se s Complications
Figure 16: Type of factor concentrates used during last 6 months
6.6.2.5 Costs
Regarding German haemophiliacs, the cost of care was covered almost 100% from the health insurance.
6.7Discussion
Haemophilia is a group of X-linked bleeding disorders affecting a considerable number of world populations. Highly purified concentrates are now available for treatment and are considered safe and effective. The main complication of treatment is the development of antibody inhibitors against either factor VIII or factor IX [1, 2]. The WFH estimates that 75% of people with haemophilia in the world do not receive adequate care [4].In developed countries, early treatment of bleeding episodes and home therapy quickly evolved as the primary management option. Presence of specialized centres, training and education of patients to achieve comprehensive haemophilia care are the main advantages of the comprehensive treatment concept in developed countries. This is not the case in most developing countries where the government does not have the resources to buy the necessary quantities of coagulation factors in the face of more urgent health priorities and hardly any patients can afford to pay for their own treatment even for on-demand home therapy. The main problems are insufficient supply with clotting factor concentrates, lack of access to these concentrates and absence of specialized care centres [6].
The Arabic region includes 22 member countries of Arab League. The Arab League is rich in natural resources, with enormous oil and natural gas resources in certain member states. Economic development in the Arab League is very disparate. Significant difference in wealth and economic conditions exist between the rich oil states like UAE, Qatar, Kuwait, on the one hand, and poor countries like Yemen, Mauritania and Djibouti on the other.
The health care provided to community, a part of which is the haemophilia care, also shows variations from country to country depending on economical level. In the Global Survey of WFH in 2009, 9702 cases of haemophilia were reported from 13 Arabic countries [3, 9]. Of 22 countries of the Arab League, only 13 have reported at least one haemophilic center. Countries such Egypt, Algeria, Tunisia, Jordan and Syria have good organized programs and more than one haemophilia center. Other countries provide a relatively weak care compared to their financial resources; for example Saudi Arabia, with a population of about 24 million, and 326 reported cases of haemophilia has only one specialized care center for haemophiliacs [3]. Factor use also varies from country to country, according to economical level. High income countries consume large quantities; for example Saudi Arabia reported the highest use of factor concentrates in the region, about 191 736 IU per PWHA [25].
Yemen is one of the poorest countries in the Arab world and its economic fortunes depend mostly on oil [67]. It is the only country of Arabic peninsula without a specialized centre for haemophilia. Taking in consideration that Yemen is a poor developing country spending about 5,6 % of its GDP on the health care, the Ministry of Health concern is focused on the major health problems of the country, such as infectious diseases and malignancies. As a result only a little or no attention is paid to rare diseases such as haemophilia. There are only two hospitals in the capital city, which offer treatment for Yemeni haemophiliacs. Thawra General Hospital and
Republican Hospital are the two largest government hospitals in the country. In either of them, there is a branch of Haematology, at the Internal Medicine Department, dealing with haemophilia cases [69].
As Yemen is a country with a large area, about 555,000 km2, and most of population
live in rural area, haemophiliacs have to cross hundreds of miles in order to seek for some care in the capital city, Sana’a. With a difficult mountainous terrain and a bad infrastructure, many of them have to travel more than 10 hours with their own cars, until they arrive in Sana’a. Under such conditions some of haemophiliacs with severe forms of the disease and life threatening complications have little chances to survive[71].
In contrast to Arabic region, haemophilia care in western European countries is a well organized process providing a qualified management to all haemophiliacs, through the Haemophilia treatment centers (HTCs) which exist in all EU Member States. Choice and levels of treatment, availability of comprehensive care and access to prophylaxis vary widely from one Member State to another [31].
In Germany, people with haemophilia are entitled to a choice of all treatments ranging from an optimal level of factor replacement therapy on demand as well as on a prophylactic basis for those who need it, to comprehensive care at a Haemophilia Treatment Centre [81]. Haemophilia care in Germany is provided through 12 specialized Haemophilia Treatment Centers. Apart from treatment and care at the HTCs, specialized physicians in practices and at haematology units in general hospitals provide care. Home treatment is available for children as well as for a number of people suffering from severe haemophilia[72].
Germany stand out for using a huge amount of FVIII IUs, about 114 333 IU per PWHA [25]. About half of the factor concentrates used in Germany are recombinant clotting factors and the other half are human plasma-derived concentrates, with the average number of units of factor VIII concentrate used per capita being 5.9 IU. About 90% of children and 40% of adult people with haemophilia in Germany receive prophylactic treatment [81].
Regarding results of the empirical study, the peak age among Yemeni haemophiliacs who participated in this study was 29 years. This fact should be attributed to two major reasons; first and most important is the fact that due to absence of care in the last few decades the patients couldn’t survive and eventually died before they reach their 30’s as a result of life threatening conditions and complications. The other reason is that the majority of patients with mild and moderate form of the disease do not seek for medical care, even if they have bleeding episodes. The Yemeni haematologists reported that they didn’t find any patient older than 30 years[71]. In the majority of Yemeni haemophiliacs, the first bleed occurred during circumcision within first 2 weeks of life, which, in many cases if not mainly, is performed at home by a nurse. In such conditions, many newborns with very low
awareness about the nature of this disease could play an important role in preventing such induced life threatening bleedings.
Currently no Yemeni patient is treated prophylactically as there is no such strategy in Yemen. The minimal care provided to severe cases is based still on FFP, Cryoprecipitate, DDAVP and even whole blood transfusions. The amount of factor concentrates used by only some severe cases with life threatening manifestations, such as ICB, is very low. According to our observation, the total amount of factor concentrates administered by 13 patients during the last 6 months was 15,050 IU, with an average of 1,157 IU per patient. Compared to Yemeni haemophiliacs, a German patient received 100,224 IU or about 90 times more factor concentrates, in addition to a good organized care with a prophylaxis program.
In Germany, the health care costs for haemophilic patients, including cost of factor replacement, were almost 100% covered by health insurance. As we mentioned in chapter 5, the majority of Yemeni population do not have a health insurance. As a result all patients, including those affected by haemophilia, should pay for every kind of care and medical procedure. The majority of haemophiliacs are not able to cover the high cost of factor concentrates, which is supposed to be covered by Ministry of Health. The cost of care among Yemeni haemophilic patients during last 6 months varied from EUR 200 EURO to EUR 2000, with an average of EUR 865 per patient. These amounts of money, which are modest in our vision, exceed the average of annual income among Yemeni population. It should be mentioned that access to factor concentrate even for severely affected Yemeni haemophiliacs is impossible[69]. As it is shown in this analysis, there is a huge difference regarding the haemophilia care provided in both regions, particularly between Yemen and Germany. The fact that other Arabic countries such as Saudi Arabia, Oman, UAE have huge financial resources and a good organized haemophilia care, is an evidence that Yemen is totally isolated and abandoned from its Arab neighbors regarding this problem.
In Yemen, as in other developing countries, the major problems with regard to haemophilia care are: absence of haemophilia centers; inadequate supply of safe factor concentrates, lack of awareness and education about the nature of the disease[6].
6.8Recommendations
After the analysis of current situation of haemophiliacs in Yemen and care provided to them, we consider that application of the following recommendations will improve the haemophilia care in this country:
• Initiation of a first step for a Haemophilia Center in Sana’a, as a result of coordination between Yemeni authorities and WFH representative for Arabic Region
• Stimulation of a humanitarian financial support, from rich Arabic neighbor countries, to found an initial center for haemophiliacs in capital city Sana’a. • Formulation of educational guidelines for haemophiliacs about the nature of
the disease
• Organization of a national network and creation of a registry for
haemophiliacs
• Home therapy (living far away from capital city, Yemeni haemophiliacs have to cross hundreds of miles to seek for some care)
• A twinning program between haemophilia centers in Germany and clinics
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